ISSN:0148-639X    

DOI:10.1002/mus.880160102

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1993

数据来源: WILEY

摘要:

AbstractIsometric quadriceps strength was remeasured in 14 healthy survivors of a group of elderly people first studied 8 years previously. There were 4 men (median age 81 years, range 79 to 84) and 10 women (82 years, range 79 to 89). They were selected for their health, not their levels of physical activity. Nevertheless, they were active when first studied and, with 1 exception, had maintained or increased their levels of physical activity. Isometric quadriceps strength was well preserved; the median change in the strength of the stronger quadriceps was only −0.3% per annum (95% confidence interval = −1.4 to +0.8). © 1993 John Wiley&Sons,

ISSN:0148-639X    

DOI:10.1002/mus.880160103

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1993

数据来源: WILEY

摘要:

AbstractWe investigated the effects of bovine brain gangliosides on the neurotoxicity of vincristine in dissociated cultures of dorsal root ganglion cells from 10‐day chick embryos. The effects of the drugs were quantified as the numbers of neurite‐bearing cells or total neurite length in individual neurite‐bearing cells. The administration of vincristine (1 to 1000 pg/mL) inhibited neurite outgrowth from the cells, whereas gangliosides (10 to 1000 m̈g/mL) protected them against this inhibition in a concentration‐dependent manner. Electron microscopy revealed vincristine‐induced fragmentation and longitudinal disorientation of microtubules in neurites and showed the protection by gangliosides against such damaging effects. Our results show that exogenous administration of gangliosides attenuates the neurotoxicity of vincristine in vitro. © 1993 John Wile

ISSN:0148-639X    

DOI:10.1002/mus.880160104

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1993

数据来源: WILEY

摘要:

AbstractElectrodiagnostic evaluation of diaphragmatic function has consisted of phrenic nerve stimulation and surface or esophageal recordings of the electrical activity of the diaphragm. Needle electromyography of the diaphragm has rarely been reported because of the perceived danger of this procedure. We describe a new technique for needle electromyography of the diaphragm. Am EMG electrode is placed in the costal insertion of the diaphragm under the 8th, 9th, or 10th rib cartilage, distant from the major vessels, pleura, lungs, and abdominal viscera. Diaphragmatic denervation was found in 42 of 81 patients using this method. There were no complications related to the procedure. Needle electromyography of the diaphragm provides important information in the diagnosis and management of respiratory dysfunction. © 1993 John Wiley&Sons, Inc

ISSN:0148-639X    

DOI:10.1002/mus.880160105

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1993

数据来源: WILEY

摘要:

AbstractPeripheral nerve dysfunction (PND) was found in as many as 43% of our patients with human T‐cell lymphotropic virus type I (HTLV‐I)–associated myelopathy (HAM/TSP). To evaluate the PND further we biopsied the sural nerve in 6 patients. The histological features were varying degrees of demyelination, remyelination, axonal atrophy and degeneration, and perineurial fibrosis. “Globule” or “sausage” formation was prominent in two of the specimens. Inflammatory infiltrates were absent. No deposits of IgG, IgM, IgA, or complement were detected in the biopsies. No viral antigen or proviral DNA was detected. It is proposed that the PND and the histological findings noted are part of HTLV‐I–associated disease and not an unrelated disorder. The pathogenesis of the PND remains unclear. There was no evidence of direct viral infection. The histological findings could represent primary changes induced by viral‐triggered release of soluble factors, such as cytokines or secondary changes to more proximal disease, e.g., root involvement. © 1993

ISSN:0148-639X    

DOI:10.1002/mus.880160106

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1993

数据来源: WILEY

摘要:

AbstractSince 1985, we prospectively followed 246 patients with ALS. The relation ship between the age of developing neurological impairment and disease duration was analyzed in 138 patients (86 men and 52 women) who died. Mean disease duration was 4.0 ± 3.8 years for men and 3.2 ± 2.5 years for women. There was an inverse, exponential, relationship between onset age and duration (goodness‐of‐fitP>0.05). Mean duration at onset age 40 years was 8.2 ± 5.0 years compared with 2.6 ± 1.4 years for patients aged 61 to 70 years (P>0.001). The ratio of young (40 years) men to women was 3.6:1. When matched for age, disease duration was the same for patients with bulbar and nonbulbar onsets. We conclude that onset age, but no sex, is the most significant predictor determining disesae duration in ALS. Longer survival in younger patients probably reflects their greater neuronal reserve. © 1993 John Wiley&S

ISSN:0148-639X    

DOI:10.1002/mus.880160107

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1993

数据来源: WILEY

摘要:

AbstractEDL muscles from normal and dystrophic(dy2j)mice of various ages were examined. Muscles were divided into three groups according to age: 7 to 14 days postnatal, 16 to 21 days postnatal, and 6 months old, to assess age and/or phenotype related differences in the muscle response to caffeine or high K+. The response of normal muscles to caffeine decreased with age and reached adult characteristics between the second and third week of postnatal life. Their response to high K+also changed during post‐natal development; specifically, the time taken to recover to 50% pretest twitch tension decreased with age, probably reflecting developmental changes in Cl−conductance. Up to 21 days of age, the sensitivity of dystrophic muscles to both caffeine and high K+was essentially similar to normal, while marked differences were observed in the adult. Taken altogether, our results suggest that while the maturation of a number of systems might be delayed in dystrophic muscles at preclinical stages of the disease, their e–c coupling and SR function (Ca2+release and reuptake) appear to be quite normal. Our results further suggest that the “abnormal” responses of dystrophic muscles at more advanced stages of the disease, when challenged by drugs acting on either of these systems, may be explained in terms of changes in muscle fiber type proportions. © 1993 John Wiley

ISSN:0148-639X    

DOI:10.1002/mus.880160108

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1993

数据来源: WILEY

摘要:

AbstractA computer model of denervation and complete reinnervation in skeletal muscle was originally developed for the purpose of furthering an understanding of the underlying mechanisms of motor unit reorganization in neurogenic diseases. We now describe its successor, a computer model for investigating different rates of denervation and reinnervation, as well as incomplete reinnervation. The new model introduces the concept of permanent denervation and features enhanced interactive control over the distribution of motor unit centers and additional measures of dispersion and co‐dispersion of muscle fibers. The use of this model for investigating pathophysiologically significant issues in denervating diseases is illustrated pathophysiologically significant issues in denervating diseases is illustrated with five different sets of parameters. These simulate some of the processes that may be operational in chronic spinal muscular atrophy, amyotrophic lateral sclerosis, and progressive postpolio muscular dystrophy. The enhanced model will allow in‐depth analysis of the influence of hypothesized pathophysiological processes on clinical, electrophysiological and pathological outcomes in human disease. © 1993 John Wiley&Sons,

ISSN:0148-639X    

DOI:10.1002/mus.880160109

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1993

数据来源: WILEY

摘要:

AbstractA 30‐year‐old man with no family history of muscle disease presented with a progressive proxirnal myopathy and calf hypertrophy characteristic of Becker muscular dystrophy. A deletion of exons 45 to 48 in the dystrophin gene was confirmed by Southern blotting and multiplex polymerase chain reaction. However, muscle biopsy showed massive accumulation of glycogen, although no significant abnormality of glycolytic pathway enzymes could be demonstrated. This patient therefore has a previously undescribed myopathy associated with both Becker muscular dystrophy and a glycogen storage disorder of unknown aetiology. © 1993 John Wiley&Sons,

ISSN:0148-639X    

DOI:10.1002/mus.880160110

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1993

数据来源: WILEY

摘要:

AbstractA 12‐year‐old girl had progressive unilateral muscle hypertrophy limited to the sole, tibialis anterior, and biceps femoris muscles. The affected muscles showed complex repetitive discharges by electromyography, necrosis and variation of fiber size upon histopathological examination, and increased metabolic activity in biochemical studies. The findings suggest a myopathic origin, but the actual cause remains unknown. © 1993 John Wiley&Sons,

ISSN:0148-639X    

DOI:10.1002/mus.880160111

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1993

数据来源: WILEY